Journal
ANNALS OF THE AMERICAN THORACIC SOCIETY
Volume 13, Issue 12, Pages 2169-2173Publisher
AMER THORACIC SOC
DOI: 10.1513/AnnalsATS.201603-207BC
Keywords
lymphadenopathy; nontuberculous; immunodeficiency; myelodysplasia; mediastinal
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Funding
- Division of Intramural Research, National Institute of Allergy and Infectious Disease, National Institutes of Health (NIH)
- National Cancer Institute, NIH [HHSN261200800001E]
- NIH
- Doris Duke Charitable Foundation
- American Association for Dental Research
- Howard Hughes Medical Institute
- Colgate-Palmolive Co.
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Rationale: Mycobacterium kansasii usually causes chronic pulmonary infections in immunocompetent patients. In contrast, disseminated M. kansasii disease is commonly associated with advanced human immunodeficiency virus infection, but is reported infrequently in other immunocompromised patients. Objectives: To identify common clinical manifestations and potential risk factors for M. kansasii infection in patients with GATA2 deficiency. Methods: We reviewed M. kansasii disease associated with GATA2 deficiency at one institution and disease associated with primary and other immunodeficiencies reported in the literature. Measurements and Main Results: Nine patients with GATA2 deficiency developed M. kansasii infections. Six patients developed disseminated disease. All patients presented with significant mediastinal lymphadenopathy or abscesses. Seven patients had pulmonary risk factors, including six smokers. The majority of patients had low numbers of neutrophils, monocytes, B cells, CD4(+) T cells, and natural killer cells. Other conditions associated with disseminated M. kansasii disease were thymic disorders and IFN-gamma/IL-12 defects. Conclusions: Disseminated M. kansasii disease involving mediastinal lymph nodes is surprisingly common in GATA2 deficiency, but also occurs in defects of IFN-gamma synthesis and response. Disseminated M. kansasii should be considered a marker indicating a need to evaluate for immunodeficiency syndromes.
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